| 產(chǎn)品編號(hào) | bsm-33188M-BF647 |
| 英文名稱 | Mouse Anti-alpha smooth muscle Actin/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的肌動(dòng)蛋白α/α-SMA/α Actin抗體 |
| 別 名 | alpha sarcomeric Actin; alpha smooth muscle Actin; Actin alpha; ASMA; ASM-A; alpha-SMA; alpha SMA; AAT6; ACTA2; Actin alpha 2 smooth muscle aorta; Actin aortic smooth muscle; ACTSA; ACTVS; Alpha 2 actin; Alpha-actin 2; Cell growth inhibiting gene 46 protein; Growth inhibiting gene 46; ACTA_HUMAN; Actin alpha 2 smooth muscle aorta; Actin aortic smooth muscle; Actin, aortic smooth muscle; Alpha 2 actin; Alpha actin 2; Alpha cardiac actin; Alpha-actin 2; Alpha-actin-2; Cell growth inhibiting gene 46 protein; Cell growth-inhibiting gene 46 protein; Growth inhibiting gene 46; MYMY5 |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 產(chǎn)品類型 | 內(nèi)參抗體 |
| 研究領(lǐng)域 | 細(xì)胞生物 發(fā)育生物學(xué) 細(xì)胞骨架 |
| 抗體來源 | Mouse |
| 克隆類型 | Monoclonal |
| 克 隆 號(hào) | 8B2 |
| 交叉反應(yīng) | Human, Mouse, (predicted: Rat, ) |
| 產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 42kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human alpha smooth muscle Actin |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein G |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background: The product encoded by this gene belongs to the actin family of proteins, which are highly conserved proteins that play a role in cell motility, structure and integrity. Alpha, beta and gamma actin isoforms have been identified, with alpha actins being a major constituent of the contractile apparatus, while beta and gamma actins are involved in the regulation of cell motility. This actin is an alpha actin that is found in skeletal muscle. Mutations in this gene cause nemaline myopathy type 3, congenital myopathy with excess of thin myofilaments, congenital myopathy with cores, and congenital myopathy with fiber-type disproportion, diseases that lead to muscle fiber defects. [provided by RefSeq, Jul 2008] Function: Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells. Subunit: Polymerization of globular actin (G-actin) leads to a structural filament (F-actin) in the form of a two-stranded helix. Each actin can bind to 4 others. Subcellular Location: Cytoplasm, cytoskeleton. Post-translational modifications: Oxidation of Met-46 by MICALs (MICAL1, MICAL2 or MICAL3) to form methionine sulfoxide promotes actin filament depolymerization. Methionine sulfoxide is produced stereospecifically, but it is not known whether the (S)-S-oxide or the (R)-S-oxide is produced (By similarity). DISEASE: Note=ACTA2 mutations predispose patients to a variety of diffuse and diverse vascular diseases, premature onset coronary artery disease (CAD), premature ischemic strokes and Moyamoya disease. Defects in ACTA2 are the cause of familial aortic aneurysm thoracic type 6 (AAT6) [MIM:611788]. AATs are characterized by permanent dilation of the thoracic aorta usually due to degenerative changes in the aortic wall. They are primarily associated with a characteristic histologic appearance known as 'medial necrosis' or 'Erdheim cystic medial necrosis' in which there is degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and an accumulation of basophilic ground substance. Defects in ACTA2 are the cause of Moyamoya disease type 5 (MYMY5) [MIM:614042]. Moyamoya disease is a progressive cerebral angiopathy characterized by bilateral intracranial carotid artery stenosis and telangiectatic vessels in the region of the basal ganglia. The abnormal vessels resemble a 'puff of smoke' (moyamoya) on cerebral angiogram. Affected individuals can develop transient ischemic attacks and/or cerebral infarction, and rupture of the collateral vessels can cause intracranial hemorrhage. Hemiplegia of sudden onset and epileptic seizures constitute the prevailing presentation in childhood, while subarachnoid bleeding occurs more frequently in adults. Defects in ACTA2 are the cause of multisystemic smooth muscle dysfunction syndrome (MSMDYS) [MIM:613834]. MSMDYS is a syndrome characterized by dysfunction of smooth muscle cells throughout the body, leading to aortic and cerebrovascular disease, fixed dilated pupils, hypotonic bladder, malrotation, and hypoperistalsis of the gut and pulmonary hypertension. Similarity: Belongs to the actin family. Database links: Entrez Gene: 101021287 Baboon Entrez Gene: 59 Human Entrez Gene: 11475 Mouse Entrez Gene: 100009271 Rabbit Omim: 102620 Human SwissProt: P62736 Human SwissProt: P62737 Mouse SwissProt: P62740 Rabbit Unigene: 500483 Human Unigene: 213025 Mouse Unigene: 195319 Rat Unigene: 3114 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 結(jié)構(gòu)蛋白（Structural Proteins） Actin α/α-Actin 是一種具有收縮能力的微絲蛋白，a-SMA廣泛分布于幾乎所有的肌型細(xì)胞中。Actin-α蛋白主要用于檢測(cè)骨骼肌、平滑肌、血管平滑肌、心肌和肌原性腫瘤 包括：平滑肌瘤、平滑肌肉瘤、橫紋肌肉瘤以及肌上細(xì)胞和肌上皮瘤。Actin（肌動(dòng)蛋白）是在所有真核細(xì)胞中都表達(dá)的高度保守的蛋白質(zhì)。它們沿微管組成了細(xì)胞骨架的主要成分。肌動(dòng)蛋白至少表達(dá)為6種異構(gòu)形式。它在心臟、骨骼橫紋肌組織和某些平滑肌組織中表達(dá)，調(diào)節(jié)其收縮功能。有報(bào)導(dǎo)說肌動(dòng)蛋白在乳房瘤中是高度磷酸化的。肌動(dòng)蛋白的功能失調(diào)也會(huì)導(dǎo)致某種類型的心臟病。平滑肌α肌動(dòng)蛋白使人更感興趣，因?yàn)榫幋a它的基因是相對(duì)局限于在血管平滑肌細(xì)胞中表達(dá)的少數(shù)幾個(gè)基因之一。肌動(dòng)蛋白是標(biāo)記平滑肌和肌上皮細(xì)胞腫瘤的有效工具。 |
